Spotting the signs and diagnosis - Addison's disease - Articles - Articles - Addison's Disease Self-Help Group ADSHG - 06 Dec 2016 08:09 PM 0 Spotting the signs and diagnosis 01 Jan 2016 10:23 PM | ADSHG in Addison's disease Addison’s disease is a rare, chronic condition brought about by failure of the adrenal glands. If untreated the condition is life threatening and unfortunately not always spotted quickly. Signs and symptoms The slowly progressive loss of cortisol and aldosterone secretion usually produces a chronic, steadily worsening fatigue, a loss of appetite, and some weight loss. Blood pressure is low and falls further when a person is standing, producing light-headedness. Nausea, sometimes with vomiting, and diarrhoea are common. The muscles are weak and often go into spasm. There are often emotional changes, particularly irritability and depression. Because of salt loss, a craving for salty foods is common. Finally, the increase in ACTH due to the loss of cortisol will usually produce a darkening of the skin that may look like an inappropriate tan on a person who feels very sick.

Unfortunately, the slowly progressive chronic symptoms are usually missed or ignored until a sudden event like a flu virus, an accident, or the need for surgery suddenly precipitates a dramatic change for the worse because of the deficient response from the adrenals to one of these stresses. This is referred to as an adrenal crisis and is a medical emergency. Diagnostic tests A medical history of the symptoms mentioned above, especially hyperpigmentation (darkening) of the skin or gums, is often enough to raise a strong suspicion, prompting the appropriate tests. For autoimmune Addison's, the patient may already have been diagnosed with one or more of the typical associated conditions: premature ovarian/gonadal failure, a thyroid condition, vitiligo, B12 deficiency, insulin-dependent diabetes or coeliac (gluten/lactose intolerance). Even where the patient themselves has none of these associated conditions, a medical history of the extended family will often identify some of these associated conditions among first and second degree relations. Quite often, however, the first clue is from the abnormal results of routine blood tests ordered by the GP or hospital.

These may include a low blood level of sodium, elevated blood level of potassium, a shift in the ratio of certain white blood cells, or surprising changes on an EKG or chest x-ray that are caused by high potassium or low blood volume. Other causes for these changes, particularly from medications, must be considered first. The ADSHG's explanatory leaflet for GPs, Diagnosing Addison's , explains the preliminary screening tests that your GP can conduct to indicate whether to refer you to a specialist for further testing. A definitive diagnosis of Addison's disease requires that definitive tests be carried out. The standard test used here is the short Synacthen test (also known as the ACTH stimulation test). The short Synacthen test is a blood test in which the amount of cortisol in the blood is measured before and after an injection of ACTH, usually over a period of 60 minutes. Individuals with adrenal disease produce a comparatively small amount of extra cortisol in response to the injection. Different hospitals apply different thresholds to define how small an increase in blood cortisol means adrenal failure. For Addison's, an elevated blood level of ACTH should also be found.

For secondary adrenal insufficiency (a pituitary cause), ACTH is likely to be abnormally low. If the patient is very sick and Addison's disease is suspected, treatment can be initiated while the diagnostic tests are being done. Once the diagnosis of adrenal insufficiency is established, causation must be explored. Serology (antibody) tests are the first step. Where these are negative, further diagnostic screens are appropriate, including a CT scan of the adrenals and screening for tuberculosis or other infections through further blood and skin tests and x-rays. Seeking medical help In the UK, you must ask your GP to refer you to an endocrinologist for specialist assessment in order for diagnosis of Addison's disease to be confirmed. It is important that all tests are carried out face-to-face under the supervision of qualified medical professionals. Our thanks to Paul Margulies, M.D., F.A.C.P., F.A.C.E., Medical Director of the United States National Adrenal Diseases Foundation and Clinical Associate Professor of Medicine, Cornell University Medical College, whose text we have adapted in this section. What is Addison’s Disease?

– Living With Addison's Disease irregular or absent menstrual periods in women, loss of interest in sex Hyperpigmentation, or darkening of the skin, can occur in Addison’s disease, although not in secondary adrenal insufficiency. This darkening is most visible on scars; skin folds; pressure points such as the elbows, knees, knuckles, and toes; lips; and mucous membranes such as the lining of the cheek. The slowly progressing symptoms of adrenal insufficiency are often ignored until a stressful event which causes them to worsen, leading to an Adrenal Crisis. Adrenal Crisis Sudden, severe worsening of adrenal insufficiency symptoms is called adrenal crisis. If the person has Addison’s disease, this worsening can also be called an Addisonian crisis. In most cases, symptoms of adrenal insufficiency become serious enough that people seek medical treatment before an adrenal crisis occurs. However, sometimes symptoms appear for the first time during an adrenal crisis. Symptoms of adrenal crisis include sudden, severe pain in the lower back, abdomen, or legs severe vomiting and diarrhea loss of consciousness If not treated, an adrenal crisis can cause death.

Addison’s Disease is caused by an autoimmune disorder, which is when the body’s immune system attacks the body’s own cells and organs. In Autoimmune Addison’s, the immune system gradually destroys the adrenal cortex—the outer layer of the adrenal glands. Primary adrenal insufficiency occurs when at least 90 percent of the adrenal cortex has been destroyed. As a result, both cortisol and aldosterone are often lacking. Sometimes only the adrenal glands are affected. Sometimes other endocrine glands are affected as well, as in Polyendocrine Deficiency Syndrome. Polyendocrine Deficiency Syndrome Polyendocrine Deficiency Syndrome is classified into type 1 and type 2. Type 1 is inherited and occurs in children. In addition to adrenal insufficiency, these children may have underactive parathyroid glands, which are four pea-sized glands located on or near the thyroid gland in the neck; they produce a hormone that helps maintain the correct balance of calcium in the body. slow sexual development. pernicious anemia, a severe type of anemia; anemia is a condition in which red blood cells are fewer than normal, which means less oxygen is carried to the body’s cells.

With most types of anemia, red blood cells are smaller than normal; however, in pernicious anemia, the cells are bigger than normal. chronic fungal infections. chronic hepatitis, a liver disease. Type 2, which is sometimes called Schmidt’s syndrome, is also thought to be inherited. Type 2 usually affects young adults and may include an underactive thyroid gland, which produces hormones that regulate metabolism slow sexual development diabetes, in which a person has high blood glucose, also called high blood sugar or hyperglycemia vitiligo, a loss of pigment on areas of the skin. How is Addison’s Disease Diagnosed? In its early stages, adrenal insufficiency can be difficult to diagnose. A health care provider may suspect it after reviewing a person’s medical history and symptoms. A diagnosis of adrenal insufficiency is confirmed through a hormonal blood test: ACTH stimulation test: The ACTH stimulation test is the most commonly used test for diagnosing adrenal insufficiency. In this test, the patient is given an intravenous (IV) injection of synthetic ACTH, and samples of blood, urine, or both are taken before and after the injection. The cortisol levels in the blood and urine samples are measured in a lab.

The normal response after an ACTH injection is a rise in blood and urine cortisol levels. People with Addison’s disease or longstanding secondary adrenal insufficiency have little or no increase in cortisol levels. Diagnosis during Adrenal Crisis: Although a reliable diagnosis is not possible during adrenal crisis, measurement of blood ACTH and cortisol during the crisis—before treatment with corticosteroids is given—is often enough to make a preliminary diagnosis. Low blood sodium, low blood glucose, and high blood potassium are also sometimes present at the time of adrenal crisis. Once the crisis is controlled, an ACTH stimulation test can be performed to help make a specific diagnosis. How is adrenal insufficiency treated? Adrenal insufficiency is treated by replacing, or substituting, the hormones that the adrenal glands are not making. The dose of each medication is adjusted to meet the needs of the patient. Cortisol is replaced with a corticosteroid, such as hydrocortisone, prednisone, or dexamethasone, taken orally one to three times each day, depending on which medication is chosen.

If aldosterone is also deficient, it is replaced with oral doses of a mineralocorticoid hormone, called fludrocortisone acetate (Florinef), taken once or twice daily. People with secondary adrenal insufficiency normally maintain aldosterone production, so they do not require aldosterone replacement therapy. During adrenal crisis, low blood pressure, low blood glucose, low blood sodium, and high blood levels of potassium can be life threatening. Standard therapy involves immediate IV injections of corticosteroids and large volumes of IV saline solution with dextrose, a type of sugar. This treatment usually brings rapid improvement. When the patient can take liquids and medications by mouth, the amount of corticosteroids is decreased until a dose that maintains normal hormone levels is reached. If aldosterone is deficient, the person will need to regularly take oral doses of fludrocortisone acetate. What problems can occur with adrenal insufficiency? Problems can occur in people with adrenal insufficiency who are undergoing surgery, suffer a severe injury, have an illness, or are pregnant. These conditions place additional stress on the body, and people with adrenal insufficiency may need additional treatment to respond and recover.

Basically anything that causes stress (good or bad) to the body needs to be mirrored with corticosteroids. Surgery: People with adrenal insufficiency who need any type of surgery requiring general anesthesia must be treated with IV corticosteroids and saline. IV treatment begins before surgery and continues until the patient is fully awake after surgery and is able to take medication by mouth. The “stress” dosage is adjusted as the patient recovers until the regular, presurgery dose is reached. Severe Injury: Patients who suffer severe injury may need a higher, “stress” dosage of corticosteroids immediately following the injury and during recovery. Often, these stress doses must be given intravenously. Once the patient recovers from the injury, dosing is returned to regular, pre-injury levels. Illness: During an illness, a person taking corticosteroids orally may take an adjusted dose to mimic the normal response of the adrenal glands to this stress on the body. Significant fever or injury may require a triple dose. Once the person recovers from the illness, dosing is then returned to regular, pre-illness levels.

People with adrenal insufficiency should know how to increase medication during such periods of stress, as advised by their health care provider. Immediate medical attention is needed if severe infections, vomiting, or diarrhea occur. These conditions can lead to an adrenal crisis. Pregnancy: Women with adrenal insufficiency who become pregnant are treated with the same hormone therapy taken prior to pregnancy. However, if nausea and vomiting in early pregnancy interfere with taking medication orally, injections of corticosteroids may be necessary. During delivery, treatment is similar to that of people needing surgery. Following delivery, the dose is gradually lessened, and the regular dose is reached about 10 days after childbirth. How is adrenal crisis treated? Adrenal crisis is treated with adrenal hormones. People with adrenal crisis need immediate treatment. Any delay can cause death. When people with adrenal crisis are vomiting or unconscious and cannot take their medication, the hormones can be given as an injection. A person with adrenal insufficiency should carry a corticosteroid injection at all times and make sure that others know how and when to administer the injection, in case the person becomes unconscious. The dose of corticosteroid needed may vary with a person’s age or size.

Some Important Points: Adrenal insufficiency is an endocrine, or hormonal, disorder that occurs when the adrenal glands do not produce enough of certain hormones. Addison’s disease, the common term for primary adrenal insufficiency, occurs when the adrenal glands are damaged and cannot produce enough of the adrenal hormone cortisol. The adrenal hormone aldosterone may also be lacking. Secondary adrenal insufficiency occurs when the pituitary gland fails to produce enough adrenocorticotropin (ACTH), a hormone that stimulates the adrenal glands to produce cortisol. If ACTH output is too low, cortisol production drops. The most common symptoms of adrenal insufficiency are chronic fatigue, muscle weakness, loss of appetite, weight loss, and abdominal pain. The slowly progressing symptoms are often ignored until a stressful event, such as surgery, a severe injury, an illness, or pregnancy, causes them to worsen. If not treated, an adrenal crisis can cause death. A diagnosis of adrenal insufficiency is confirmed through hormonal blood and urine tests. Imaging studies of the adrenal and pituitary glands can be useful in helping to establish the cause. Adrenal insufficiency is treated by replacing, or substituting, the hormones that the adrenal glands are not making.

Problems can occur in people with adrenal insufficiency who are undergoing surgery, suffer a severe injury, have an illness, or are pregnant. These conditions place additional stress on the body, and people with adrenal insufficiency may need additional treatment to respond and recover. People with adrenal insufficiency should always carry identification stating their condition, “adrenal insufficiency,” in case of an emergency, as well as the supplies necessary to administer an emergency corticosteroid injection. Sinéad x Addisons Disease | Skin Pigmentation Skin Pigmentation Contact Addisons Disease Addison’s disease is a rare, chronic condition brought about by the failure of the adrenal glands which produce essential hormones such as cortisol. Lifelong, continuous treatment with steroid replacement therapy is required. With the right balance of daily medication, most people with the disease are able to continue life much as it was before their illness. One of the symptoms of this disease is increased pigmentation of the skin, however, this is not usually linked to Addison’s Disease until a minor infection has an abnormally long recovery time and the cause investigated.

Addison's Disease | Diseases and Their Cures Wikia | Fandom powered by Wikia Diseases and Their Cures Wikia Edit Addison’s disease is a rare, chronic condition brought about by the failure of the adrenal glands. Lifelong, continuous treatment with steroid replacement therapy is required. With the right balance of daily medication, most people with the disease are able to continue life much as it was before their illness. Addison’s disease is a chronic condition that is brought upon by the failure of adrenal glands. The adrenal glands are at the top of the kidneys, and produce adrenalin, also known as the “fight or flight” stress hormone. The glands are made up of two parts. The core, or medulla, is surrounded by the outer shell, or cortex. The medulla is the part that produces adrenalin, and is not detrimental to Addison’s disease. The cortex through is critical, because it produces the steroid hormones that are essential to human life. The hormones produced are cortisol and aldosterone, Patients with Addison’s disease require life-long treatment, but can often return to living life close to normally. How does Addison's Disease Affect the Endocrine System? Edit Addison’s disease affects the endocrine system through lack of needed hormones.

The endocrine system revolved around the production and control of hormones, therefore a lack in an essential hormone throws off the system. The body relies of the hormones produced by the adrenal glands, and an insufficient amount is dangerous to both the body and the system. To clarify, the hormones that are in the Endocrine system are: estrogen, progesterone, steroids, cortisol, and cortisone, and chemicals such as adrenalin (epinephrine), norepinephrine, and dopamin. Signs and Symptoms Depression Body hair loss or sexual dysfunction in women There are certain signs a person can identify to see if they have Addison’s disease. These symptoms can be things such as, but not limited to extreme fatigue, weight loss, low blood pressure, decreased appetite, and hyperpigmentation. The patient may also undergo a craving for salt, body hair loss, muscle or joint pains, and sometimes even fainting. Diagnosis Edit Your doctor will talk to you first about your medical history and your signs and symptoms. If your doctor thinks that you may have Addison's disease, you will have to take these tests; Blood Test, ACTH stimulation test, Insulin-induced hypoglycemia test, and lastly imaging tests.

When diagnosing Addison’s disease, your doctor will talk to the patient about their medical history and symptoms. If the doctor believes that the patient has the Addison’s, then they may be subject to certain tests such as a blood test, an ACTH stimulation test, or an insulin-induced hypoglycemia test. The blood test helps the doctor to grasp an indication of whether the signs and symptoms may be caused by adrenal insufficiency. The ACTH stimulation test involves measuring the level of cortisol in the patient’s blood before and after injecting ACTH. The ACTH notifies the adrenal glands to produce cortisol, and if the cortisol levels do barely or do not rise, then the adrenal glands are damaged. As for an insulin-induced hypoglycemia test, the patient’s blood sugar and cortisol levels are measured after injecting various intervals of insulin. If the glucose levels do not fall and the cortisol levels do not increase, then the patient’s cause of adrenal insufficiency may be a pituitary disease. Treatment Edit All treatment for Addison's disease involves hormone replacement therapy to correct the levels of steroid hormones your body isn't producing.

Some methods of treatment are; Oral corticosteroids, Corticosteroid injections To treat Addison’s disease, one must go through a hormone replacement therapy. This therapy will attempt to correct the levels of steroid hormones that the patient’s body is not producing. Different treatment options include things such as oral corticosteroids, which are a type of steroid hormones produced in the adrenal cortex of vertebrates, or corticosteroid injections. The injections are mostly reserved for patients who are suffering from vomiting and cannot hold down the oral medications. Addison’s Disease Causes - What Leads to Adrenal Insufficiency? Addison’s Disease Causes Written by Daniel J. Toft MD, PhD | Reviewed by Robert M. Sargis MD, PhD Adrenal insufficiency—when your adrenal glands don’t produce enough of the hormone cortisol—can be caused by a primary adrenal gland disorder (this is called Addison’s disease or primary adrenal insufficiency). Alternatively, adrenal insufficiency can be caused by a deficiency of the adrenocorticotropin hormone (ACTH). ACTH stimulates the adrenal glands to produce cortisol, so if there’s too little ACTH, you may have low levels of cortisol (this is called secondary adrenal insufficiency).

Much more rare—but still a possible cause of adrenal insufficiency—is an injury to the hypothalamus, a gland in the brain that produces releasing and inhibiting hormones (hormones that tell the other endocrine glands to start and stop production). A hypothalamic injury can lead to a corticotrophin-releasing hormone (CRH) deficiency. CRH is what tells the pituitary gland to produce ACTH, which then stimulates cortisol production in the adrenal glands. A CRH deficiency can lead to an ACTH deficiency—and then to adrenal insufficiency. Primary Adrenal Insufficiency: Addison’s Disease The outer layer of the adrenal glands is called the adrenal cortex. If the cortex is damaged, it may not be able to produce enough cortisol. A common cause of primary adrenal insufficiency is an autoimmune disease that causes the immune system to attack healthy tissues. In the case of Addison’s disease, the immune system turns against the adrenal gland(s). There are some very rare syndromes (several diseases that occur together) that can cause autoimmune adrenal insufficiency. Autoimmune Polyendocrine Syndrome Autoimmune polyendocrine syndrome (APS) is a rare cause of Addison’s disease.

Sometimes referred to as multiple endocrine deficiency syndrome, APS is further categorized as type 1 or type 2. APS type 1. Symptoms start during childhood. Almost any organ can be affected by autoimmune damage. Fortunately it is extremely rare—only several hundred cases have been reported worldwide. Conditions associated with APS type 1 include: Addison’s disease Delayed or slow sexual development Vitamin B12 malabsorption / deficiency (pernicious anemia) Candidiasis (chronic yeast infection) Hepatitis APS Type 2. Symptoms primarily develop in adults aged 18 to 30. Conditions associated with APS type 2 include: Addison’s disease Underactive or overactive thyroid function Delayed or slow sexual development Diabetes Celiac disease When people with APS type 2 are affected by Addison’s disease and thyroid disease, this condition is sometimes called Schmidt’s syndrome.

Other causes of primary adrenal insufficiency include: Amyloidosis—protein build up in organs (very rare) Bleeding into the adrenal glands Cancer that spreads to the adrenal glands Infections (bacterial, fungal, tuberculosis) of the adrenal glands Surgical removal of the adrenal glands Secondary Adrenal Insufficiency: ACTH Deficiency Secondary adrenal insufficiency is caused by a lack of the adrenocorticotropin hormone (ACTH). ACTH is made in the pituitary gland. It’s your brain’s signal to the adrenal glands, telling them to make cortisol. By far the most common cause of this type of adrenal insufficiency is chronic steroid use. When steroids (which have the same action as cortisol) are taken to treat other medical conditions, the brain stops sending the ACTH signal to the adrenals to make the body’s own cortisol. Once steroids are stopped, it can take a long time for the brain to resume sending this signal, which causes adrenal insufficiency.

The following conditions can also affect ACTH production and therefore hinder cortisol secretion: Loss of blood flow (circulation) to the pituitary gland Pituitary tumor, or other brain tumor that puts pressure on the pituitary Radiation treatment of pituitary tumor Surgical removal of the pituitary gland or hypothalamus Severe head injury Addison’s Disease: When It Becomes a Crisis The symptoms of Addison’s disease may slowly develop and progress. Sometimes a stressful event or illness causes symptoms to suddenly develop or worsen. Adrenal crisis—or Addisonian crisis—is a condition that merits urgent medical attention because these symptoms can be fatal. The symptoms of an Addisonian crisis include: Dehydration and/or severe vomiting and diarrhea Stabbing pain in the abdomen, low back, or legs Low blood pressure (shock) Low blood sugar Loss of consciousness Life-saving treatment with steroids can only be given if the emergency medical team knows that you have adrenal insufficiency; the symptoms of Addisonian crisis can affect your thinking and even cause loss of consciousness, so it’s essential that all patients with adrenal insufficiency wear a medical alert bracelet or necklace that clearly states their diagnosis.

Addison's Disease - NORD (National Organization for Rare Disorders) Subdivisions of Addison’s Disease No subdivisions found General Discussion Addison's disease is a rare disorder characterized by inadequate production of the steroid hormones cortisol and aldosterone by the two outer layers of cells of the adrenal glands (adrenal cortex). The symptoms of classic Addison's disease, also known as primary adrenal insufficiency, result from the insufficient production of these two hormones, cortisol and aldosterone. Major symptoms include fatigue, gastrointestinal abnormalities, and changes in skin color (pigmentation). Behavior and mood changes may also occur in some individuals with Addison's disease. Increased excretion of water and low blood pressure (hypotension) can lead to extremely low concentrations of water in the body (dehydration). The symptoms of Addison's usually develop slowly, but sometimes can develop rapidly, a serious condition called acute adrenal failure. In most cases, Addison's disease occurs when the body's immune system mistakenly attacks the adrenal glands causing slowly progressive damage to the adrenal cortex. Signs & Symptoms The symptoms of Addison’s disease can vary from one individual to another. Symptoms usually develop slowly over time and are usually vague and common to many conditions (nonspecific).

This often leads to delays in the proper diagnosis of Addison’s disease. In rare cases, the symptoms of Addison’s disease can develop rapidly causing a condition called acute adrenal failure. Fatigue is the most common symptom of Addison’s disease. Another common initial symptom of Addison’s disease is the development of patches of skin that are darker than the surrounding skin (hyperpigmentation). This discoloration most commonly occurs near scars, by skin creases such as the knuckles, and on the mucous membranes such as the gums. Skin abnormalities can precede the development of other symptoms by months or years, but do not occur in every person. Some individuals with Addison’s disease may also develop a condition called vitiligo in which white patches may appear on different areas of the body. This may vary from one or two small spots on the skin or multiple, larger affected areas. Black freckles may develop on the forehead, face or shoulders in some cases. A variety of gastrointestinal symptoms may be present including nausea, vomiting, and abdominal pain. Diarrhea is less common, but may also occur. Affected individuals may have a poor appetite and unintentional weight loss and may develop progressive fatigue and muscle weakness.

Muscle pain (myalgia), muscle spasms and joint pain may also occur. Dehydration can also affect individuals with Addison’s disease. An additional symptom that may occur is low blood pressure (hypotension), which can cause lightheadedness or dizziness upon standing. Temporary loss of consciousness (syncope) can occur in some cases. Addison’s disease can also lead to changes in emotion and behavior. The disorder has been associated with irritability, depression, and poor concentration. Individuals with Addison’s disease may have cravings for salt or salty foods and low blood sugar (glucose) levels. Women with Addison’s disease may have irregular menstrual periods, lose body hair and have a decreased sexual drive. In some cases, symptoms of Addison’s disease may appear suddenly, a condition called acute adrenal failure or an addisonian crisis. During an addisonian crisis, affected individuals may develop a sudden loss of strength; severe pain in the lower back, abdomen or legs; vomiting and diarrhea potentially causing dehydration; and low blood pressure and loss of consciousness. An addisonian crisis is a medical emergency that can cause life-threatening complications such as shock or kidney failure if not treated.

A crisis is usually set off when affected individuals are under stress such as during an accident, trauma, surgery or severe infection. Causes Most cases of Addison’s disease occur due to damage or destruction of the adrenal cortex, the outermost layers of the adrenal glands (zona fasciulata, which secretes cortisol and zona glomerulosa, which secretes aldosterone). Symptoms usually do not develop until 90 percent of the adrenal cortex has been damaged. When Addison’s disease is caused by the inability of the adrenal cortex to produce hormones such as cortisol and aldosterone, it is referred to as primary adrenal insufficiency. When the adrenal glands are undamaged, but cannot produce these hormones for other reasons such as due to abnormalities of the pituitary gland, the condition is referred to as secondary adrenal insufficiency (For more information on secondary adrenal insufficiency see the Related Disorders section below). In approximately 75 percent of cases of Addison’s disease (primary adrenal insufficiency), damage to the adrenal cortex results from an autoimmune reaction. For reasons not completely understood, the body’s natural immune defenses (antibodies, lymphocytes, etc.) mistakenly attack healthy tissue, in this case healthy cells of the adrenal gland.

Autoimmune Addison’s disease may occur by itself (as an isolated condition) or as part of a large disorder specifically the autoimmune polyendocrine syndromes I (APS type-1) and II (Schmidt syndrome). In the past, tuberculosis was the major cause of Addison’s disease and still remains a major cause of the disorder in developing countries. Less common causes of Addison’s disease include repeated infections especially fungal infections, the spread of cancer from another area of the body to the adrenal glands, bleeding (hemorrhaging) into the adrenal gland, and the abnormal accumulation of a fatty-like substance with the adrenals (amyloidosis). (For more information on these disorders, choose “tuberculosis” and/or “amyloidosis” as your search terms in the Rare Disease Database.) Addison’s disease occurs due to failure of the adrenal glands to produce sufficient amounts of the hormones, cortisol and aldosterone. Hormones are chemicals produced by glands that control and regulate certain activities of cells or organs of the body. The characteristic symptoms of Addison’s disease result from low levels of cortisol and aldosterone in the body. Cortisol affects how the body responds to stress and is released in greater quantities when a person is under stress.

Cortisol has many additional functions in the body including helping to maintain blood pressure and cardiovascular function, helping to regulate the amount of water in the body, playing a role in controlling blood sugar levels by balancing the effects of insulin and assisting in proper immune system function and in the breakdown (metabolism) of carbohydrates, fats and proteins. Aldosterone affects the sodium and potassium ion equilibrium (electrolyte imbalance) in the body, as well as helping to maintain water levels and, therefore, blood pressure and blood volume. Deficiency of aldosterone hinders the kidney’s ability to filter salt and water, resulting in low blood pressure. The adrenal glands also produce androgen, a steroid hormone that controls the development of certain secondary sexual characteristics such as hair growth. Deficiency of androgen can cause loss of body hair and diminished sex drive in women. In males, androgen is primarily produced in the testes, not the adrenal glands. Therefore, males do not express signs of decreased androgens as Addison’s disease is an adrenal disease. In rare cases, Addison’s disease has run in families suggesting that, in these cases, individuals may have a genetic predisposition to developing the disorder.

A genetic predisposition means that a person may carry a gene or gene(s) for a disease but the disease may not be expressed unless other factors (such as something in the environment) trigger the disease. Affected Populations Addison’s disease affects males and females in equal numbers. Approximately 1 in 100,000 people in United States have Addison’s disease. The overall prevalence is estimated to be between 40 and 60 people per million of the general population. Because cases of Addison’s disease may go undiagnosed, it is difficult to determine its true frequency in the general population. Addison’s disease can potentially affect individuals of any age, but usually occurs in individuals between 30-50 years of age. Addison’s disease was first identified in the medical literature in 1855 by a physician named Thomas Addison. Related Disorders Symptoms of the following disorders can be similar to those of Addison’s disease. Comparisons may be useful for a differential diagnosis. ACTH deficiency is a rare disorder that arises as a result of decreased or absent production of adrenocorticotropic hormone (ACTH) by the pituitary gland. It is a form of secondary adrenal insufficiency.

A decline in the concentration of ACTH in the blood leads to a reduction in the secretion of adrenal hormones, resulting in adrenal insufficiency (hypoadrenalism). Adrenal insufficiency leads to unintentional weight loss, lack of appetite, weakness, nausea, vomiting, and low blood pressure (hypotension). ACTH deficiency is a disorder that usually starts during adulthood, although a few cases have begun during childhood. Low blood levels of sugar and sodium and high potassium levels (hypoglycemia, hyponatremia and hyperkalemia) also occur. The pituitary hormone ACTH may be undetectable in blood tests, and the level of the adrenal hormone cortisol is abnormally low. Some adrenal hormones that are decreased include cortisol and aldosterone. Hormones that are precursors of male sex hormones known as “androgens” may also be reduced. Although males with this disorder usually have a normal hair pattern, females have very little pubic and underarm (axillary) hair. In contrast to Addison’s disease, skin pigmentation usually remains normal. Emotional and behavioral changes may occur as well.

(For more information on this disorder, choose “ACTH deficiency” as your search term in the Rare Disease Database.) Congenital adrenal hyperplasia (CAH) is a group of disorders resulting from defective synthesis of the corticosteroid hormones of the adrenal gland. The adrenal gland becomes enlarged. The adrenal gland produces “male” sex hormones (androgens) in both males and females because these are overproduced in certain forms of CAH. The external genitals of some females with this disorder become masculinized to various degrees. Lack of glucocorticoids, especially cortisol, causes various problems. Lack of mineralocorticoids, primarily aldosterone, causes salt and water imbalances which may be life threatening. (For more information on this disorder, choose “Adrenal Hyperplasia” as your search term in the Rare Disease Database.) Adrenoleukodystrophy is an X-linked recessive genetic disorder caused by an abnormality in the ABCD1 gene on the X chromosome. This condition affects the white matter of the nervous system and the adrenal cortex.

Some affected individuals have adrenal insufficiency, which means that reduced amounts of certain hormones such as adrenaline and cortisol are produced, leading to abnormalities in blood pressure, heart rate, sexual development and reproduction. Some of those affected experience serious neurological problems that can affect mental function and lead to disability and reduced life span. This condition has been categorized into six types based on symptoms and age of onset: childhood cerebral ALD, adolescent cerebral ALD, adrenomyeloneuropathy, adult cerebral ALD, adrenal insufficiency only and ALD that occurs in females.(For more information on this disorder, choose “adrenoleukodystrophy” as your search term in the Rare Disease Database.) Autoimmune polyendocrine syndrome type II, also known as Schmidt syndrome, is a rare autoimmune disorder in which there is a steep drop in production of several essential hormones by the glands that secrete these hormones. When first described, this disorder was thought to involve only adrenal insufficiency (Addison’s disease) and thyroid insufficiency (Hashimoto’s thyroiditis). However, over time, as more patients were studied, the scope of the disorder was expanded to include disorders of other underperforming endocrine glands.

These include the gonads, which secrete sex hormones; the pancreas which secretes insulin and is intimately tied up with diabetes mellitus; and sometimes the parathyroid glands. Failure of the endocrine glands to function is usually accompanied by signs of malnutrition because the ability of the intestinal tract to absorb nutrients is reduced dramatically. Since the combination of affected glands differs from patient to patient, the signs of this disorder are diverse. Most cases of this disorder are sporadic although some clinical researchers believe that there is a familial or hereditary trait associated with AIPS-II. If so, it may involve a complex interaction among many genes. (For more information on this disorder, choose “Schmidt syndrome” as your search term in the Rare Disease Database.) Autoimmune polyendocrine syndrome type I, (APS type 1) also known as APECED syndrome, is a rare genetic syndrome involving the autoimmune system. It is a combination of several distinct disorders and is defined as the subnormal functioning of several endocrine glands at the same time (concurrently). The acronym APECED stands for Autoimmune Polyendocrinopathy (APE), Candidiasis (C) and Ectodermal Dysplasia (ED).

Autoimmune disease affecting one gland is frequently followed by the impairment of other glands. In this syndrome two major patterns of failure have been described. Beginning in childhood, yeast infections of either the mouth or nails are usually one of the first apparent symptoms of APS type-. Low plasma levels of calcium and phosphate (hypoparathyroidism) are often diagnosed before adrenal dystrophy. There may be an inability to adequately absorb nutrients with resulting diarrhea. Anemia, autoimmune thyroid disease, and loss or delay of sexual development may also occur. The combination of ectodermal dysplasia with candidiasis or hypoparathyroidism may also indicate an APS type-1 patient. In addition to hypoparathyroidism, lack of tooth enamel (enamel dystrophy), loss of hair (alopecia) and absence of pigment in areas of the skin (vitiligo) may also present. An eye disorder, corneal dystrophy (keratopathy), may also occur. There may be failure of sexual development, yeast infections of the mouth and nails (candidiasis), and the inability to properly absorb nutrients from food (malabsorption). Patients may develop liver disease or insulin- dependent diabetes as well as any combination of the various disorders.

(For more information on this disorder, choose “APS type-1” as your search term in the Rare Disease Database.) Diagnosis A diagnosis of Addison’s disease is suspected based upon a thorough clinical evaluation, a detailed patient history and identification of characteristic findings. Often a diagnosis is made incidentally during a routine exam when a blood test shows low levels of sodium or high levels of potassium. A diagnosis may be confirmed through a variety of specialized tests including the ACTH stimulation test, insulin-induced hypoglycemia test and x-ray examination. The ACTH stimulation test measures the amount of cortisol in the blood. During this test, adrenocorticohormone (ACTH) is injected into the body, stimulating production of cortisol. If the test fails to stimulate adequate cortisol production, it indicates that the adrenal glands are damaged or not functioning properly. The insulin-induced hypoglycemia test may be used to determine if the symptoms of Addison’s disease are due to problems with the pituitary gland. This test measures blood sugar (glucose) levels before and after the injection of fast-acting insulin, which should lead to a drop in glucose and a rise in cortisol.

X-ray imaging techniques such as computed tomography (CT scan) of the abdomen may be taken to check the size of the adrenal glands and to detect other signs of disease such as calcification of the adrenal glands. During a CT scan, a computer and x-rays are used to create a film showing cross-sectional images of an organ’s tissue structure. Standard Therapies Treatment The treatment of Addison’s disease is directed toward the specific symptoms that are apparent in each individual. Treatment may require the coordinated efforts of a team of specialists. Individuals with Addison’s disease are treated by replacing the deficient steroid hormones (cortisol and aldosterone). Cortisol is replaced by the drug hydrocortisone and aldosterone is replaced by the drug fludrocortisone. The dosage of these drugs is different for each individual and the dosage may be increased during infection, trauma, surgery and other stressful situations to prevent an acute adrenal crisis. Individuals should be encouraged to increase the salt intake in their diets.

An adrenal crisis demands immediate hormonal investigation and intravenous (injected directly into a blood vessel) administration of high-dose hydrocortisone and fluid (salt water) and electrolyte replacement; a short-term course of other drugs called vasopressors may be needed to maintain blood pressure. Affected individuals should carry a card or wear a tag stating that they have Addison’s disease. Investigational Therapies Some individuals with Addison’s disease may also be treated with androgen replacement therapy, such as testosterone or androstenedione hormone. Researchers have reported improvements in mood, fatigue and general psychological well-being among individuals with Addison’s disease treated with androgen replacement therapy. For women, androgen replacement therapy may also improve their sex drive and sexual satisfaction. Information on current clinical trials is posted on the Internet at . All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office: Tollfree: (800) 411-1222 Natural Remedies for Addison's Disease See also companion article Adrenal support Addison's disease is a rare, chronic condition brought about by the failure of the adrenal glands, it is not usually diagnosed until well progressed, symptoms can include fatigue, weakness, loss of weight, faintness, low blood pressure, nausea, vomiting, salt cravings and painful muscles and joints. The adrenal glands have an inner core known as the medulla and an outer shell known as the cortex. The inner medulla produces adrenaline, and the cortex produces steroid hormones essential for life - cortisol and aldosterone. Cortisol mobilises nutrients, enables the body to fight inflammation, stimulates the liver and helps to control the amount of water in the body. Aldosterone regulates salt and water levels. Around 70% of Addison's cases are auto-immune related, where antibodies attach to cells in the adrenal cortex. These destroy the cells which make cortisol and aldosterone.

The adrenal medulla is not affected, in which case, we would recommend that you read The Auto Immune Disorder Protocol See also companion article Adrenal support For non auto-immune cases, you should be guided by The Core Regime Physicians in China may prescribe Liquorice alone or with cortisol to treat mild cases of Addison's disease, in which the body produces too little of this hormone. Disclaimer: Regenerative Nutrition advocates a holistic approach to natural health and wellbeing. The body's ability and power to heal depends upon the totality of diet, nutrition, lifestyle and environmental factors. No claims for the cure of any disease is intended, or implied. Always consult a health care practitioner when combating disease states. The statements in this article have not been approved by the FDA. Removal of Blocks to Health, Healing and Wellness We have defined the major blocks to health improvement which should be considered the number one priority for resolution before one goes on to a more specific healing regimen such as that detailed in the programme above. By removing these blocks one will feel stronger and more prepared for a specific programme and will achieve far greater success with it. Please read this essential article here The Core Regime Not all of the remedies listed here will neccessarily be needed.

We strive to bring you the most effective remedies to resolve your health problems in the simplest supplemental regime possible. We aim to treat the actual root causes of health problems holistically rather than supply remedies that will give temporary or partial relief from only the symptoms. This has been our mission since the founding of Regenerative Nutrition in 1994. As we uncover more fundamental truths about the causes, and therefore the relief, of diseases and health problems we update and revise our product range and recommendations. Above, we detail targeted remedy recommendations for Addison's Disease, but we further suggest that you view the core regime article and supplement pages by clicking here To achieve overall good health and long term resolution of this health problem the remedies selected here should be combined with core regime remedies for the best results. It may well be that there is some overlap and that many of the remedies in the core regime are listed here also. If you need any assistance in selecting the most suitable remedies, or have any questions, please Contact Us Supplements for Addison's Disease The supplements recommended for the treatment of Addison's Disease are listed below. Not all will necessarily be discussed in the article, as some may have been added after the article was posted.

If a listed supplement is not mentioned within the article, or you wish to know more about any of the listed supplements, you can find out more information by clicking on that supplement below. You can also now add to your basket directly from this page. STORY By: Sarah J. Baker Wednesday, 04. December 2002 "Addison’s disease is a rare, chronic condition brought about by the failure of the adrenal glands. Lifelong, continuous treatment with steroid replacement therapy is required. With the right balance of daily medication, most people with the disease are able to continue life much as it was before their illness. ... Adrenal function The adrenal glands sit at the top of the kidneys, one on each side of the body and have an inner core (known as the medulla) surrounded by the outer shell (known as the cortex). The inner medulla produces adrenaline, the “fight or flight” stress hormone. While the absence of the adrenal medulla does not cause disease, the cortex is more critical. It produces the steroid hormones that are essential for life: cortisol and aldosterone.

Cortisol mobilises nutrients, it enables the body to fight inflammation, it stimulates the liver to produce blood sugar and it also helps control the amount of water in the body. Aldosterone regulates salt and water levels which affect blood volume and blood pressure. The adrenal cortex also produces sex hormones known as adrenal androgens; the most important of these is DHEA. The normal adrenal cortex has an enormous functional reserve. This is called upon by the body especially in times of intense stress, such as surgery, trauma or serious infection. One of the most significant consequences of Addison’s disease is, therefore, the body’s failure to adapt to such stresses and, in the absence of adequate steroid cover, this may result in a state of shock, known as an Addisonian crisis, which is a medical emergency. Causes of adrenal failure Thomas Addison first identified the disease in 1855 while working at Guy’s Hospital in London. At that time, the main cause of the disease was as a complication of tuberculosis. TB still remains an important cause of Addison’s in Third World countries. HIV (AIDS) is now becoming another significant infectious disease causing adrenal failure among third world population.

In more affluent countries, the most frequent cause of Addison’s disease is destructive atrophy whereby an over-active immune system starts attacking the body’s own organs; in this case, the adrenals. This accounts for around 70% of all cases and affects more women than men. In common with most other autoimmune diseases, the exact reason for the atrophy is unknown. Other, much rarer causes of Addison’s include certain fungal infections, adrenal cancer and adrenal haemorrhage (for example, following a car accident). Symptoms Addison’s disease is not usually apparent until over 90% of the adrenal cortex has been destroyed, so that very little adrenal capacity is left. This can take months to years and is known as primary adrenal insufficiency. Symptoms of the disease, once advanced, can include severe fatigue and weakness, loss of weight, increased pigmentation of the skin, faintness and low blood pressure, nausea, vomiting, salt cravings and painful muscles and joints. Because of the rather non-specific nature of these symptoms and their slow progression, they are often missed or ignored until, for example, a relatively minor infection leads to an abnormally long convalescence which prompts an investigation.

Frequently, it is not until a crisis is precipitated that attention is turned to the adrenals. Secondary failure Secondary adrenal insufficiency is sometimes described as “Addison’s”, although it has a very different cause. It mostly occurs when a pituitary tumour (such as an adenoma) forms, although autoimmune destruction of the pituitary gland is also known. In secondary adrenal insufficiency, the pituitary gland no longer triggers the adrenals to produce cortisol, and DHEA production is also believed to decline. In most cases of secondary adrenal insufficiency, however, aldosterone is still produced, as its production is stimulated by other hormonal regulatory systems. The pituitary hormone which triggers cortisol production is called ACTH; it is responsible for the extra pigmentation found in primary Addison’s. People with secondary adrenal failure do not experience the extra pigmentation found in primary Addison’s, because their ACTH levels are declining. Long term use of high doses of steroid drugs to treat other illnesses (for example high–dose prednisone for bowel disease or asthma) can also cause temporary or permanent loss of adrenal function. This is often referred to as secondary adrenal suppression.

Until the development of steroid medication in the late 1940s, the outcome of adrenal disease was invariably fatal. With the development of modern steroid medications, individuals with Addison’s disease can expect to have a fairly normal life span, provided they manage their daily medication sensibly. People with Addison’s must always be aware of their own health and ready to increase their dosage if they get sick or are seriously injured." [ ][Addison's Disease Self Help Group] Welcome to the Facebook Addison's Support Community! This support group enables people affected by Addison's Disease, family members, friends and medical professionals a place to meet, interact, and share ideas with each other. Please note that message posted here should NOT be taken as medical advice but only opinion and experience of patients or caregivers. For medical diagnosis and/or treatment, please consult your personal physician. Understanding Addison's Disease -- the Basics Understanding Addison's Disease Understanding Addison's Disease -- the Basics Understanding Addison's Disease Treatments What Is Addison's Disease? One way the body keeps itself in balance is by using chemical messengers called hormones to regulate various functions. Just above each of your kidneys is a small adrenal gland. These glands make hormones essential to a healthy life.

When they don't make enough of these hormones, Addison's disease is the result. Addison's disease is a rare condition. Only one in 100,000 people has it. It can happen at any age to either men or women. People with Addison's disease can lead normal lives as long as they take their medication . President John F. Kennedy had the condition. In Addison's disease, the adrenal glands don't make enough of a hormone called cortisol, or less often, a related hormone called aldosterone. That's why doctors sometimes call the illness ''chronic adrenal insufficiency,'' or hypocortisolism. Cortisol's most important function is to help the body respond to stress. It also helps regulate your body's use of protein, carbohydrates, and fat; helps maintain blood pressure and cardiovascular function; and controls inflammation. Aldosterone helps your kidneys regulate the amount of salt and water in your body -- the main way you regulate blood volume and keep your blood pressure under control. When aldosterone levels drop too low, your kidneys cannot keep your salt and water levels in balance. This makes your blood pressure drop. There are two forms of Addison's disease.

If the problem is with the adrenal glands themselves, it's called primary adrenal insufficiency. If the adrenal glands are affected by a problem starting somewhere else -- such as the pituitary gland -- it's called secondary adrenal insufficiency. What Causes Addison's Disease? Most cases of Addison’s disease result from a problem with the adrenal glands themselves (primary adrenal insufficiency). Autoimmune disease accounts for 70% of Addison’s disease. This occurs when the body's immune system mistakenly attacks the adrenal glands. This autoimmune assault destroys the outer layer of the glands. Long-lasting infections -- such as tuberculosis , HIV , and some fungal infections -- can harm the adrenal glands. Cancer cells that spread from other parts of the body to the adrenal glands also can cause Addison's disease. Continued Less commonly, Addison's disease is due to secondary adrenal insufficiency caused by a problem with the pituitary gland or a problem with the hypothalamus, both located in the center of the brain . These glands produce hormones that act as a switch and can turn on or off the production of hormones in the rest of the body. A pituitary hormone called ACTH is the switch that turns on cortisol production in the adrenal gland.

If ACTH levels are too low, the adrenal glands stay in the off position. Another cause of secondary adrenal insufficiency is prolonged or improper use of steroid hormones such as prednisone . Less common causes include pituitary tumors and damage to the pituitary gland during surgery or radiation . WebMD Medical Reference Reviewed by Jennifer Robinson, MD on March 01, 2015 Sources

Question: Addison's Disease is a rare chronic disorder brought about by the failure of which glands?
Answer: ADRENAL GLANDS